Update on Addison

Addison is doing much better. She still has a long way to go, but she is taking baby steps in the right direction. She has been off nitric oxide for over 24 hours now. She was started on an oral (or technically g-tube) medication that has the same effect on her lungs as the nitric oxide and they increased the dosage of that today. She is still intubated and likely will be for at least a few more days. They discontinued her paralytic yesterday, but she is still sedated. She has woken up off and on and is moving a little bit. Since she is no longer paralyzed, she was also able to get rid of the continuous EEG. She was extremely swollen from all the fluids they gave her, so she’s on a diuretic drip; that has helped a lot. Her foley catheter also came out yesterday. She is still on an antibiotic. She has been getting her home feeds (minus a little water) and tolerating them well. She was started on a course of steroids yesterday. She has ranged from 40 to 60% oxygen. They made some minor changes to her ventilator settings yesterday and had to increase her oxygen as a result, but overall she has tolerated them. They want to keep her oxygen at 50% or lower today. Her chest x-ray this morning was back to looking normal. Her wonderful nurse yesterday gave her a bath and got as much of the glue out of her hair as she could. Violet sent Addi a hair clippy the other day, so she has that in. (Because when you have three girls, hair accessories are very important; they can make or break your day).

Now, I’ll try (and probably fail miserably) to explain why all this happened and what they have diagnosed Addison with. I’m still trying to understand everything myself, so I’ll just hit the highlights. They think she had components of both brochopulmonary dysplasia (BPD) and pulmonary hypertension going on. From what the intensivist says, they are recently finding out that there are two types of BPD. The first type is what Violet had, where the ventilator and oxygen do damage to the alveoli. The second type is apparently what Addison has. In this type, the alveoli are not as damaged and they typically look like those in a normal, healthy newborn, but there is inflammation in the lungs. It is frequently occult for a while and shows up later on. She had an echocardiogram that was normal, but since she needed the nitric oxide, the doctor says that means she has pulmonary hypertension or at least some type of pulmonary vascular reactivity (treated the same). She was started on a steroid yesterday to help with the inflammation. Apparently both of these problems are something she will outgrow. Most children on this medication for pulmonary hypertension are off of it by the time they start kindergarten. The most important things for Addi are to: keep her healthy and avoid exposure to illness as much as possible; make sure she’s getting enough calories and growing well (which she has been); and prevent any type of aspiration. They have been questioning whether she has had some silent aspiration. She has been off her reflux medication for almost a year because she stopped throwing up and was doing well without it. She has been placed on a reflux medication in the hospital and I’m sure will have to continue it at home.

I’m glad to have some answers. She got very sick very fast and it was terrifying. It’s frustrating that we just saw a pulmonary doctor less than a month ago, and she said Addison’s lungs were fine. And apparently, at that time there was no way to tell that they weren’t. It just seems a little crazy that something so serious was able to sneak up on us and all the specialists she’s seen (not to mention all the time we have spent in the PICU over the past year). I’m thankful that she’s moving in the right direction. She is taking baby steps, but they are baby steps towards getting better. She’s doing everything right, we just have to let her take her time. Again, thank you all for your thoughts, prayers, encouragement and offers of help. It means a lot.